■内脏利什曼病(VL)和巨噬细胞活化综合征(MAS)的组合由于其相似的临床表现而使诊断困难,预后不良,特别是因为治疗方法仍然缺乏编码。我们报告了一名来自Berkane的17个月大女性患者的病例,有3个月的无政府性发热病史,伴有由脸色苍白组成的贫血综合征和由鼻出血组成的出血性综合征。体格检查显示温度为39°C,下四肢水肿,皮肤和粘膜苍白,牙龈瘀斑,漂白的头发,和肝脾肿大.
■全血细胞计数显示全血细胞减少伴深度再生型正常细胞性贫血,浓度为3g/dL,白细胞4860/mm3,中性粒细胞减少680/mm3,血小板减少12.000/mm3,血涂片未见异常。这些异常与低白蛋白血症有关,高甘油三酯血症,高铁蛋白血症,乳酸脱氢酶(LDH)水平为337IU/L,低凝血酶原时间(PT)为56%,纤维蛋白原水平为1g/L。直接Coombs试验为阳性。脊髓造影检查显示存在利什曼原虫体和噬血细胞图。诊断为与MAS相关的内脏利什曼病。患者接受脂质体两性霉素B和皮质类固醇治疗,临床和生物进化良好,治疗耐受性良好。
■VL和MAS的关联仍然很少,即使在非地方病区也应引起,因为晚期诊断会使预后恶化,甚至可能导致患者的死亡,尽管进行了积极的治疗。
UNASSIGNED: The combination of visceral leishmaniasis (VL) and macrophage activation syndrome (MAS) makes the diagnosis difficult due to their similar clinical presentation, with a poor prognosis especially since the treatment is still poorly codified.We report the
case of a 17-month-old female patient from Berkane, presenting for a 3 months history of anarchic fever with anemic syndrome made up of pallor and hemorrhagic syndrome made up of epistaxis. Physical examination revealed a temperature of 39 ° C, lower limbsedema, paleness of skin and mucous membranes, gingival petechiae, bleached hair, and hepatosplenomegaly.
UNASSIGNED: The complete blood count showed pancytopenia with deep aregenerative normochromic normocytic anemia at 3 g/dL, leukocytes were at 4860/mm 3 with neutropenia at 680/mm 3 and thrombocytopenia at 12.000/mm3, the blood smear was without abnormality. These anomalies were associated with a hypoalbunemia, hypertriglyceridemia, hyperferritinemia, lactate dehydrogenase (LDH) level was at 337 IU/L, low prothrombin time (PT) at 56 % and fibrinogen level at 1 g/L. The direct Coombs test was positive. Examination of the myelogram revealed the presence of leishmania bodies and figures of hemophagocytosis. A diagnosis of visceral leishmaniasis associated with MAS was made.The patient was put on liposomal amphotericin B and corticosteroid therapy with good clinical and biological evolution and good therapeutic tolerance.
UNASSIGNED: The association of VL and MAS remains rare and should be evoked even in non-endemic areas since late diagnosis worsens the prognosis and may even be responsible for the death of patients despite an aggressive treatment.